Superior Semicircular Canal Dehiscence (SSCD) Diagnosis

[Andrea Rings]

Greetings,

Last Wednesday I was diagnosed with a rare medical condition: Superior Semicircular Canal Dehiscence (SSCD) of both ears.

When I developed pulsatile tinnitus (heartbeat sounds) on top of the non-pulsatile tinnitus (ringing), my local ENT laughed it off and told me I would simply have to learn to live with these symptoms as well as the hyperacusis (sound sensitivity), aural fullness (ear pressure), and autophony (hearing myself blink). I decided to seek the help of an ENT at the University of Pennsylvania (Penn Medicine in Philadelphia). After several MRIs, MRVs, and MRAs, this doctor quickly ruled out aneurysms, tumors, and blood vessel disorders.

On Wednesday, I went in for a CAT scan of my temporal bones. A temporal bone CAT scan is a limited kind of head scan that focuses on the lower part of the skull and soft tissues. They're used in patients with hearing loss, chronic ear infections, and middle and inner ear diseases. This scan revealed the reason for my symptoms. The bony structures above my inner ears have holes/tears/openings (i.e. dehiscence) that should not be there. These gaps make the blood flow (pulsing) in my brain audible and create a situation of conductive hearing loss, which brings about the other symptoms. In most people, this top (superior) semicircular bone in the ear creates a seal between the ear and the brain. I lack that seal.

There is a surgery, a craniotomy, to repack the bony openings, but my doctor does not recommend it for me at this time. Usually this surgery is a go-to for those who suffer with the symptom of vertigo. Since I don't experience vertigo (and hopefully never will) the risks of the surgery do not outweigh the potential gains. He will monitor my hearing and wants me back yearly to have hearing tests. He also wants to see me if I develop any new symptoms or if my symptoms worsen.

I am relieved that I don't have a tumor, aneurysm, or blood vessel disorder, and I am praying that this condition isn't progressive. SSCD syndrome is a fairly new diagnosis - only discovered in 1998 by a Johns Hopkins ENT. My ENT said there is no way to predict if I will worsen over time. Though this is not exactly good news for me, I am very grateful to have an answer. These strange symptoms have been causing me distress for nearly a year and a half.

(N.B. My tinnitus began on August 3, 2019 in the right ear and September 29, 2019 in the left ear. I noticed the hyperacusis shortly after the ringing started. The pulsatile tinnitus began in April 2020 - 8 months after the non-pulsatile tinnitus started.)

I wish us all peace and health.

 

[Drachen]

Hi there, Andrea!

I am terribly, terribly sorry to hear about this diagnosis you've received. It's already bad enough that you were previously afflicted by tinnitus, something that can rarely be conclusively evaluated in terms of cause, but now you have this additional problem on top of everything else. The fact that the only treatment option appears to be a surgery is certainly not comforting.

With hopes, this will not worsen over time, but if that is the case, you will have the surgery to consider. Either way, I similarly wish you peace and health. You should not have to deal with this.

 

[Orions Pain]

Hi Andrea, I'm sorry you're suffering with this but happy you've at least managed to establish a diagnosis.

I have a question. Is your pulsatile tinnitus and autophony constant 24/7, or situational/intermittent? I can also hear myself blink but weirdly not all the time, and have moments of pulsatile tinnitus on top of hyperacusis and regular tinnitus.

 

[Matchbox]

At least there's a fix out there for you, and a doctor that gave a crap enough to follow up with figuring out the "why" instead of the live with it part.

 

[Andrea Rings]

Hi there, Andrea!

I am terribly, terribly sorry to hear about this diagnosis you've received. It's already bad enough that you were previously afflicted by tinnitus, something that can rarely be conclusively evaluated in terms of cause, but now you have this additional problem on top of everything else. The fact that the only treatment option appears to be a surgery is certainly not comforting.

With hopes, this will not worsen over time, but if that is the case, you will have the surgery to consider. Either way, I similarly wish you peace and health. You should not have to deal with this.

Thank you very much for your very kind and compassionate response. This forum has been so helpful to me over the last year - in large part because of people like you. It is very comforting to be able to chat with others who truly know what the tinnitus experience is like.

No one should have to deal with this - neither you, nor me, nor anyone! I am optimistic about the five new drugs that are currently going through clinical trials. Hopefully a cure (or at least, an effective treatment) is on the horizon.

You're right when you say that the surgery is something to consider if the condition exacerbates, and I do consider myself very fortunate to have insight into the etiology of my tinnitus.

I see you're a new sufferer. The onset is a very trying time for most. Are you doing okay? When will you be evaluated?

 

[Andrea Rings]

Hi Andrea, I'm sorry you're suffering with this but happy you've at least managed to establish a diagnosis.

I have a question. Is your pulsatile tinnitus and autophony constant 24/7, or situational/intermittent? I can also hear myself blink but weirdly not all the time, and have moments of pulsatile tinnitus on top of hyperacusis and regular tinnitus.

Thank you for your kind note. Yes, I feel fortunate to have the diagnosis. Knowing why can definitely help someone cope with such rattling symptoms.

To answer your questions, my pulsatile tinnitus and autophony are intermittent but frequent. Generally, day-to-day noise and goings-on often drown it out. I hear it the most when in bed, especially (and always!) if I'm on my side - which incidentally also makes the non-pulsatile tinnitus much louder, too. I've learned to sleep on my back with a pink noise machine and a weighted blanket to calm my nerves. My hyperacusis is constant too, but I only have the pain with certain higher-pitched noises, like dishes clanking together. High volume only hurts when the sound source is proximal to my ear. If my husband speaks too closely to my ear, I have pain - but if I am several feet away from him as he speaks, I am fine.

The non-pulsatile tinnitus is bilateral, asymmetrical, and constant. I used to dream of hearing silence again, but at this point I have habituated and I don't notice it unless I think about it. Hypnotherapy helped me to habituate to the screeching train break/tea kettle sirens in my ears.

Are you pursuing an evaluation? For me, the fifth time (that is, fifth ENT) was a charm.

 

[Andrea Rings]

At least there's a fix out there for you, and a doctor that gave a crap enough to follow up with figuring out the "why" instead of the live with it part.

You are correct, and I don't take for granted that I finally found an answer. It took a while for me to find an ENT who truly listened and was well trained enough to know what to look for. Every time I heard "learn to live with it," I was devastated. It appears that your tinnitus began a year after mine did. How are you coping? I wish you luck as you investigate the etiology. May your providers be as astute and compassion as my fifth ENT was. I remain hopeful that a cure or treatment will soon be found.

 

[Orions Pain]

Thank you for your kind note. Yes, I feel fortunate to have the diagnosis. Knowing why can definitely help someone cope with such rattling symptoms.

To answer your questions, my pulsatile tinnitus and autophony are intermittent but frequent. Generally, day-to-day noise and goings-on often drown it out. I hear it the most when in bed, especially (and always!) if I'm on my side - which incidentally also makes the non-pulsatile tinnitus much louder, too. I've learned to sleep on my back with a pink noise machine and a weighted blanket to calm my nerves. My hyperacusis is constant too, but I only have the pain with certain higher-pitched noises, like dishes clanking together. High volume only hurts when the sound source is proximal to my ear. If my husband speaks too closely to my ear, I have pain - but if I am several feet away from him as he speaks, I am fine.

The non-pulsatile tinnitus is bilateral, asymmetrical, and constant. I used to dream of hearing silence again, but at this point I have habituated and I don't notice it unless I think about it. Hypnotherapy helped me to habituate to the screeching train break/tea kettle sirens in my ears.

Are you pursuing an evaluation? For me, the fifth time (that is, fifth ENT) was a charm.

Gotcha. Yeah even if a diagnosis doesn't necessarily have an easy cure I've found that mentally it helps a bit knowing your symptoms are all recognized!

But wow that's pretty much identical to mine. My pulsatile tinnitus is also most audible when I'm laying down, same with the eye clicking. Sometimes the swooshing gets really intense when I drink water or strain my neck. It's mostly in one ear, but the non-pulsatile tinnitus is bilateral.

I had a CT scan over the summer and I thought it would scan my ears but I think they only scanned my sinuses? Not sure if both those areas fall under the same scan but my GP ordered this so she probably had no idea of what to even look for. I saw two ENTs fall of 2019 with no help.

Do you have any TTTS (muscle spasms in the ears)?

 

[Drachen]

Thank you very much for your very kind and compassionate response. This forum has been so helpful to me over the last year - in large part because of people like you. It is very comforting to be able to chat with others who truly know what the tinnitus experience is like.

No one should have to deal with this - neither you, nor me, nor anyone! I am optimistic about the five new drugs that are currently going through clinical trials. Hopefully a cure (or at least, an effective treatment) is on the horizon.

You're right when you say that the surgery is something to consider if the condition exacerbates, and I do consider myself very fortunate to have insight into the etiology of my tinnitus.

I see you're a new sufferer. The onset is a very trying time for most. Are you doing okay? When will you be evaluated?

Absolutely! It was my pleasure, and I am very interested in trying to help others or provide support wherever I can. As you mentioned, this is a problem that no one should have to suffer, and I wish more was being done to address and potentially remedy it. These new drugs are certainly promising, and I hope that they will actually prove effective and marketable. Their success could also mean there is more incentive to look into further treatments and cures.

As for me, well I would not say I am doing okay, but I am at least fortunate to be in a "functional" state. I am still going about my life as if there is a film of grime covering everything. I feel as if I have become a different, much sadder and less fulfilled person ever since my day of onset.

I have indeed been evaluated at this point, so I should probably update my profile accordingly. I have been to four medical professionals now: two primary care doctors, one audiologist, and one ENT. None of them could give me a conclusive diagnosis. I must then consider the most likely causes, which appears to be either noise-induced hearing loss or ototoxicity. I am starting to consider other options as well such as TMJ problems, but I have no associated pain in that respect. As I'm sure you knew before, not knowing the answers of the etiology add an entire other realm of frustration to this matter.

 

[Andrea Rings]

As for me, well I would not say I am doing okay, but I am at least fortunate to be in a "functional" state. I am still going about my life as if there is a film of grime covering everything. I feel as if I have become a different, much sadder and less fulfilled person ever since my day of onset

My heart goes out to you. You are in the toughest stage right now. It does get better, so hang in there. A hypnotherapist who specializes in training the sub-conscience to regard the tinnitus as a benign body noise really helped me lift that film of grime. I would be happy to share her contact info. There are also tinnitus counselors who see clients via Zoom who may help. Lastly, I compiled a list of articles and YouTube videos that I personally found helpful. A year and a half later, I still read/watch them. These sources helped me to keep hope alive. I would be happy to share these links as well. Let me know. In the meantime, know you are not alone.

Peace,
Andrea

 

[Gstar2020]

Hi Andrea,

Thank you for your post. Your symptoms sound a little like mine.

Just a quick question for you please?

Is your ringing tinnitus also linked to the SSCD?

I've seen quite a few GPs, ENT consultants and a Neuro surgeon but I still don't have a clear certain diagnosis.

I developed pulsatile tinnitus followed by the ringing tinnitus, followed by eye floaters but I've been told these are not linked in any way. I find it a little hard to believe though.

Kind regards.

 

[Swampy]

Gotcha. Yeah even if a diagnosis doesn't necessarily have an easy cure I've found that mentally it helps a bit knowing your symptoms are all recognized!

But wow that's pretty much identical to mine. My pulsatile tinnitus is also most audible when I'm laying down, same with the eye clicking. Sometimes the swooshing gets really intense when I drink water or strain my neck. It's mostly in one ear, but the non-pulsatile tinnitus is bilateral.

I had a CT scan over the summer and I thought it would scan my ears but I think they only scanned my sinuses? Not sure if both those areas fall under the same scan but my GP ordered this so she probably had no idea of what to even look for. I saw two ENTs fall of 2019 with no help.

Do you have any TTTS (muscle spasms in the ears)?

Heya!

Your pulsatile tinnitus sounds very similar to mine. I've had pulsatile tinnitus and many other ear conditions (various tinnitus sounds, Patulous Eustachian Tubes, sinus issues, TTTS/MEM, cholesteatoma etc) for years. But since I had 2 operations for other issues to stiffen my eardrums a couple years back, the pulsatile tinnitus lessened. Until last year I noticed it creeping back in slightly but only when I was stretching my neck.

Until, this recent month, it's started getting intense out of nowhere sometimes, but mostly with neck movements or swallowing water!

I had a CT scan last October and MRI (although the pulsatile tinnitus wasn't as bad as it is now then), and it's all clear, so unsure why this was suddenly get intense.

Do you have any ideas for why yours is that way?

Hope you are well

 

[FGG]

You are correct, and I don't take for granted that I finally found an answer. It took a while for me to find an ENT who truly listened and was well trained enough to know what to look for. Every time I heard "learn to live with it," I was devastated. It appears that your tinnitus began a year after mine did. How are you coping? I wish you luck as you investigate the etiology. May your providers be as astute and compassion as my fifth ENT was. I remain hopeful that a cure or treatment will soon be found.

Have you looked into the newer outpatient surgery at all? It's apparently a viable alternative to craniotomy and they may be willing to consider it in non-vertigo cases:

Outpatient Procedure Repairs Superior Semicircular Canal Dehiscence

 

[Autumnleaves]

I've suffered from severe pulsatile tinnitus since last August. In the beginning I thought I was hallucinating. I had to go on medication for anxiety. It sounded like an amplifier cranked up, a bass drum and a helicopter in my ear/head 24/7. Medication only relieved it for a couple of hours. I went to countless doctors before being referred to an ear specialist.

Long story short, I have superior canal dihescense syndrome. I was about to have surgery this month until they did a CTA/CTV scan. My awesome luck showed a 4.5 mm intracranial carotid artery aneurysm. I'm so hopeless and depressed over this. I'm on hold with ear surgery since surgeon won't do surgery with an aneurysm. Still waiting to see what is going to be done about aneurysm. Neurosurgeon believes it's small.

Don't give up and think there isn't an underlying reason for pulsatile tinnitus. You have to be your own advocate.

 

[Mike O]

My awesome luck showed a 4.5 mm intracranial carotid artery aneurysm

I feel your pain and hope you get some good news with this. My luck seems to be similar; get a test done and find a new issue. I too had an issue with my carotid artery, some plaque had ruptured and was blocking it off and was rushed into surgery. Just stay positive, nowadays the surgeons can do some amazing things.